Liver transplantation for erythropoietic protoporphyria with hepatic failure: a case report

Erythropoietic protoporphyria (EPP) is a disorder of heme synthesis in which deficient ferrochelatase activity leads to excessive production and biliary excretion of protoporphyrin. The main clinical features--photosensitivity and hepatobiliary disease that may progress to liver failure--are caused by the toxicity of protoporphyrin. Orthotopic liver transplantation is an effective treatment of liver failure caused by EPP. In this report we have described an EPP Chinese man with end-stage liver disease. He was successfully transplanted. A 3-year follow-up study of protoporphyrin levels, liver tests, and liver biopsies showed no EPP recurrence after liver transplantation.     

Benefits of chronic plasmapheresis and intravenous heme-albumin in erythropoietic protoporphyria after orthotopic liver transplantation

Erythropoietic protoporphyria (EPP) is characterized by a deficiency of ferrochelatase the final enzyme of the heme biosynthetic pathway. Patients with EPP may overproduce protoporphyrin IX, chiefly in developing erythrocytes. In some, protoporphyrin accumulates and causes toxicity, particularly to the skin and liver. Orthotopic liver transplantation (OLT) treats the severe liver disease that sometimes occurs in EPP; however, it does not correct the underlying metabolic disorder. We recently reported a patient with EPP who was improved with plasmapheresis and i.v. heme-albumin before OLT. Subsequently he developed histological and biochemical evidence of recurrent hepatotoxicity from protoporphyrin in the graft liver. We now report successful treatment of the patient with additional plasmapheresis and heme-albumin with improvement of hepatic histological and biochemical abnormalities. We conclude that plasmapheresis and heme-albumin are of benefit in EPP complicated by hepatotoxicity before and after liver transplantation.     

The impact of recurrent glomerulonephritis on graft survival in recipients of human histocompatibility leucocyte antigen-identical living related donor grafts.

BACKGROUND: Graft loss due to rejection is uncommon after human histocompatibility leukocyte antigen-identical living related donor (LRD) transplantation, resulting in an excellent long-term graft survival. Data on the impact of recurrence of the original disease on graft survival after LRD transplantation are scarce. METHODS: We have studied the influence of recurrent glomerulonephritis in adult recipients of a human histocompatibility leukocyte antigen-identical LRD graft transplanted in our center in the period from 1968 to 1996. To that end, the data of 33 patients with proven or suspected primary glomerulonephritis and 27 patients with nonglomerular diseases were analyzed. RESULTS: The patient survival was similar in both groups at 5, 10, and 20 years. The functional graft survival, i.e., graft survival after censoring for death, was, however, significantly worse for patients with glomerulonephritis as underlying disease (P<0.01). At 5 years graft survival was 100% vs. 88%, at 10 years 100% vs. 70%, and at 20 years 100% vs. 63%, respectively. Thus none of the patients with nonglomerular diseases lost a graft, whereas eight grafts were lost in the group of patients with glomerulonephritis. The main cause of graft loss in this patient group was recurrent glomerulonephritis (n=5), whereas chronic vascular rejection caused graft loss in two patients and occlusion of a transplant artery was the cause in one. A clinically significant proteinuria was detected in six more patients in the glomerulonephritis group: a recurrent glomerulonephritis was diagnosed in four patients and in two patients there was no biopsy. The cumulative incidence of recurrence was as high as 45% at 12 years after transplantation. CONCLUSION: Recipients of a human histocompatibility leukocyte antigen-identical LRD kidney have a good prognosis with respect to graft survival. After censoring for death, recurrent glomerulonephritis is the main cause of graft failure in these patients and the impact of recurrent disease on graft survival will become even more prominent with longer follow-up.     

Impact of donor age and year of transplantation on graft and patient survival following liver transplantation for hepatitis C virus

BACKGROUND: Hepatitis C virus (HCV) infection has become the most common indication for liver transplantation (LT). Graft and patient survival are adversely affected by recurrent infection of the graft. Recent publications have described an inferior outcome for recently transplanted HCV patients and have highlighted the impact of advancing donor age on severity of recurrent HCV. The donor age at which a measurable impact on graft and patient outcome can be observed has not clearly been defined. In addition, the impact of donor age on graft and patient survival for non-HCV patients needs to be examined. METHODS: We have examined a large European liver transplant database to define the impact of transplantation date and donor age on graft and patient survival for HCV patients (n = 4,736) and the impact for a comparison group of transplanted alcoholic liver disease patients (ALD, n = 5,406). RESULTS: For the entire cohorts, graft and patient survival of HCV patients was inferior to ALD patients. Since 1987, there has been a steady and ongoing improvement in the outcome of transplanted ALD patients, an improvement not observed for HCV patients. Every year since 1989, there has been an increase in liver donor age. Graft and patient survival for both ALD and HCV cohorts was adversely affected by advancing donor age. Comparison of graft and patient survival for HCV and ALD cohorts was made according to donor age (donor age subgrouped <20, 20-30, 30-40, 40-50, 50-60 and >60 years of age). For donors younger than 40 years of age, HCV and ALD recipient graft and patient survival are not significantly different. For donors older than 40, HCV recipient graft survival is inferior to ALD graft survival, an inferiority that increases for each advancing decade of donor age. For donors older than 50 years, HCV recipient patient survival is inferior to ALD patient survival, an inferiority that increases when the donor age is greater than 60 years. CONCLUSION: The results of liver transplantation for European HCV patients is inferior to a comparison group of ALD patients, and have not improved during the past 15 years. Liver donor age has increased significantly during that period. Advancing donor age has an adverse influence on graft and patient survival for ALD and HCV patients, but a significantly greater impact is observed for HCV patients when the donor is older than 40 years.     

Post-transplant lymphoproliferative disorders: clinicopathological analysis of 43 cases in a single center, 1990-2009

Post-transplant lymphoproliferative disorders (PTLDs) are a heterogeneous set of complications of organ transplantation associated with poor patient prognosis. We analyzed the clinicopathological features of PTLDs in 43 adult and pediatric recipients of solid organ or bone marrow transplantation at a large transplant service in the Republic of Korea between 1990 and 2009. Of 4545 solid organ and 747 bone marrow transplant recipients, 37 (0.81%) and 6 (0.8%), respectively, developed heterogeneous types of PTLDs. The cumulative incidences of PTLDs during this period were 1.79% (4/223) for heart transplant recipients, 0.78% (17/2192) for kidney transplant recipients, and 0.77% (16/2067) for liver transplant recipients. The patterns of disease onset, histology, and patient survival were associated with the types of organs transplanted. There is a trend for shorter overall survival (OS) in recipients with early-onset PTLDs and monomorphic PTLD histology, while kidney transplant recipients showed favorable OS. This study may be the first comprehensive analysis of the characteristics of PTLDs in Korean patients.     

Pediatric Liver Transplant Recipients Who Undergo Transfer to the Adult Healthcare Service Have Good Long‐Term Outcomes

Liver transplantation has transformed survival for children with liver disease necessitating the transfer of a growing number of patients to the adult healthcare service. The impact of transfer on outcomes remains unclear. The aim of this single‐center study of 137 consecutive pediatric liver transplant recipients was to examine the effect of transfer on patient and graft survival. The median time from transplant to transfer was 10.4 years and the median age of the patients at transfer was 18.6 years. After transfer, there were 5 re‐transplants and 12 deaths in 14 patients. The estimated posttransfer 10‐year patient and graft survival was 89.9% and 86.2%, respectively. Overall, 4 patients demonstrated graft loss as a consequence of chronic rejection. Graft loss was associated with older age at first transplant (p = 0.008). When compared to young adult patients transplanted in the adult center, the transferred patients did not have inferior graft survival from the point of transfer (HR 0.28; 95% CI 0.10–0.77, p = 0.014). This suggests that transfer did not impact significantly on graft longevity. In conclusion, pediatric liver transplant recipients who undergo transfer to the adult service have good long‐term outcomes.     

Liver transplantation in children with sickle-cell disease.

Severe liver disease is an unusual but potentially fatal complication of sickle-cell disease (SCD). Liver transplantation has been complicated by ongoing SCD and thrombosis. We reviewed 214 pediatric transplants done at our institution from 1990 to 2005. Three patients were transplanted for complications of SCD, including intrahepatic cholestasis and viral hepatitis. Overall patient and graft survival was 66%. One patient died after 6 years from a subdural hematoma. There were not any incidences of graft loss, primary nonfunction, or thrombosis. All 3 patients required between 1 and 4 postoperative transfusions to keep hemoglobin (Hgb) >9 g/dL with an S fraction of less than 25%. One patient required a preoperative transfusion for a hemoglobin S (HbS) fraction of 30%. Mean follow-up has been 4.2 years (range, 2.6-5.4 years). All 3 children continued to suffer sequelae from their SCD. One child suffered from recurrent sickle-cell hepatopathy and chronic graft failure. In conclusion, children with SCD can in rare instances develop acute and chronic liver failure. These children can be successfully transplanted with good outcomes. Careful attention must be paid to HbS fraction and hemoglobin level to prevent sickling and vascular thrombosis. Unfortunately, liver transplant cannot alter the natural course of the disease.     

Caroli's disease and outcomes after liver transplantation.

Caroli's disease is a rare autosomal recessive disorder characterized by intrahepatic cystic dilatation of the bile ducts that, when progressive, leads to intrahepatic stones, recurrent cholangitis, portal hypertension, cholangiocarcinoma, and liver failure. Liver transplantation is a promising curative option for advanced Caroli's disease. The aim of this study was to determine the outcomes of liver transplantation in unselected patients with Caroli's disease and recommend an evidence-based therapeutic algorithm for the management of Caroli's disease. Of the 78,124 patients transplanted in the United States between 1987 and 2006, 104 had Caroli's disease; 96 of these underwent liver alone, and 8 underwent combined liver/kidney transplantation. The patient survival and graft survival were analyzed by Kaplan-Meier survival analysis, and risk of death and risk of graft loss were analyzed by Cox proportional hazards regression. The overall 1-, 3-, and 5-year graft (79.9%, 72.4%, and 72.4%) and patient (86.3%, 78.4%, and 77%) survival rates were excellent for patients after liver transplantation. For combined liver/kidney transplantation (n = 8), the 1-year patient survival and graft survival were 100%. Proportional hazards analysis identified Asian ethnicity, elevated bilirubin, requirement of life support or hospitalization prior to transplantation, and a cold ischemia time greater than 12 hours as associated with increased risk of both graft loss and death. A history of prior transplant or prior abdominal surgery was also associated with increased risk of graft loss. In conclusion, liver transplantation is an excellent treatment option for patients with advanced Caroli's disease and should be considered in a timely fashion to prevent worsening complications including refractory cholangitis and cholangiocarcinoma.     

Liver transplantation for primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease that progresses to end-stage liver disease. This report is a retrospective analysis of a Canadian centre experience with liver transplantation (LT) for PSC. Of 1107 LTs performed between 1984 and 2002, 132 were performed on 111 patients with PSC. Patient survival at 1, 3, 5, and 10 years was 84.5%, 84.5%, 83.4%, and 68.9%, respectively. Graft survival at 1, 3, 5, and 10 years was 80.8%, 79.8%, 72.7%, and 55.3%. These were not significantly different from overall patient survival (P =.91) or graft survival (P =.28) in non-PSC patients transplanted over the same time period. Early mortality was predominantly related to primary nonfunction and multi-organ failure; late mortality was predominantly related to malignancy. No patient with known cholangiocarcinoma (CCA) underwent LT, but three patients had an incidental CCA noted on explant pathology. All three died of widespread metastatic disease (10.8, 38.0, and 39.8 months after LT). Nineteen patients lost their primary grafts requiring retransplantation, and two of these patients required a third transplant. Recurrent PSC was detected in six patients and suspected in another six. Four patients have been retransplanted for recurrent PSC. Chronic rejection was detected in nine patients. Eight have required retransplantation. The incidence of biliary complications was 16.2%. CONCLUSIONS: LT is effective therapy for PSC. Patient and graft survival is comparable to that seen in patients transplanted for indications other than PSC, but long-term graft survival may be lower. Recurrent PSC and chronic rejection are the major determinants of graft loss.     

Older donor livers show early severe histological activity, fibrosis, and graft failure after liver transplantation for hepatitis C

BACKGROUND: In hepatitis C virus (HCV)-positive liver transplant recipients, infection of the allograft and recurrent liver disease are important problems. Increased donor age has emerged as an important variable affecting patient and graft survival; however, specific age cutoffs and risk ratios for poor histologic outcomes and graft survival are not clear. METHODS: A longitudinal database of all HCV-positive patients transplanted at our center during an 11-year period was used to identify 111 patients who received 124 liver transplants. Graft survival and histological endpoints (severe activity and fibrosis) of HCV infection in the allografts were compared as a function of donor age at transplantation. RESULTS: By Kaplan-Meier analyses, older allografts showed earlier failure and decreased time to severe histological activity and fibrosis as compared with allografts from younger donors. By Cox proportional hazards analysis, older allografts were at greater risk for all severe histologic features and decreased graft survival as compared with younger allografts (P< or =0.02 for all outcomes). Analysis of donor age as a dichotomous variable showed that donors greater than 60 yr were at high risk for deleterious histologic outcomes and graft failure. An age cutoff of 60 yr showed a sensitivity of 94% and specificity of 67% for worse graft survival by receiver operating characteristics curve. CONCLUSIONS: Advanced donor age is associated with more aggressive recurrent HCV and early allograft failure in HCV-positive liver transplant recipients. Consideration of donor age is important for decisions regarding patient selection, antiviral therapy, and organ allocation.     

Patient and graft outcomes following liver transplantation for sarcoidosis

End-stage liver disease as a consequence of hepatic sarcoidosis is a rare indication for liver transplantation. Consequently, there is a paucity of information on the pre-transplant findings and post-operative course of individuals transplanted for hepatic sarcoidosis. The purpose of this study was to evaluate our experience with liver transplantation for sarcoidosis. Cases were identified by review of the Mount Sinai Hospital liver transplant database. For each case, two control patients with other causes of liver failure matched for age, gender and date of transplant were selected for comparison. Hepatic sarcoidosis was the indication for liver transplantation in only seven of 2117 (0.3%) adult transplants performed from September 1988 to June 2004. The diagnosis of sarcoidosis was established by findings of extensive, non-caseating granulomas in pre-transplant liver biopsy specimens or in the native liver explant. Extrahepatic disease was limited to pulmonary involvement in four patients. Sarcoid cases were statistically more likely to have diabetes mellitus (100% vs. 21%, p = 0.001) and less likely to have antibodies to hepatitis C (0% vs. 50%, p = 0.047). Rates of acute cellular rejection were 57% in cases and 36% in controls (p = 0.397). Hepatic granulomas were identified in one patient at 5.6 yr of follow-up. Among cases, the 1-yr graft and patient survival rates were 100% and 5-yr graft and patient survival rates were 86%. The 1- and 5-yr graft and patient survival rates were comparable with those of patients transplanted for other indications.     

Outcome of pediatric renal transplantation in Labfi Nejad Hospital, Tehran, Iran

Kidney transplantation is the treatment of choice for children with end-stage renal disease. In Iran, a kidney transplantation program was started in the Labfi Nejad Hospital, Tehran in 1985. From 1985 to 2003, 278 children (mean age 11.6 years, 59.7% males) received their first renal transplant. All transplants were donations from live donors (12.5% live-related donors); 30.8% of patients were preemptively transplanted. The overall 1-year patient survival rate was 92% and the 5-year survival rate 74%. The median graft survival time was 7.2 years. The rate of graft survival was 88.8% at 1 year, 77% at 3 years, 67% at 5 years, 50% at 7 years, and 43% at 10 years after transplantation. The survival rate of patients and transplants improved significantly with time (p<0.05). In patients transplanted before 1997, the 5-year graft survival was 50% and 82% in patients transplanted after 1997. At the same time intervals, the frequency of acute rejection episodes was 66.6 versus 40.8% and of chronic rejection 50.5 versus 28.7%. The outcome in children below the age of 6 years was poor. Graft survival was negatively correlated with the frequency and an early time point of acute rejection episodes. The modus of transplantation (preemptive or postdialysis) did not influence the results. In conclusion, patient and graft survival in transplanted children significantly improved with time, thus reflecting greater medical and surgical experience, new immunosuppressive drugs, and better compliance.     

Solid Organ Transplantation in AL Amyloidosis

Vital organ failure remains common in AL amyloidosis. Solid organ transplantation is contentious because of the multisystem nature of this disease and risk of recurrence in the graft. We report outcome among all AL patients evaluated at the UK National Amyloidosis Centre who received solid organ transplants between 1984 and 2009. Renal, cardiac and liver transplants were performed in 22, 14 and 9 patients respectively, representing <2% of all AL patients assessed during the period. One and 5‐year patient survival was 95% and 67% among kidney recipients, 86% and 45% among heart recipients and 33% and 22% among liver recipients. No renal graft failed due to recurrent amyloid during median (range) follow up of 4.8 (0.2–13.3) years. Median patient survival was 9.7 years among 8/14 cardiac transplant recipients who underwent subsequent stem cell transplantation (SCT) and 3.4 years in six patients who did not undergo SCT (p = 0.01). Amyloid was widespread in all liver transplant recipients. Solid organ transplantation has rarely been performed in AL amyloidosis, but these findings demonstrate feasibility and support a role in selected patients.     

Erythropoietic protoporphyria in an adult with sequential liver and hematopoietic stem cell transplantation: A case report

Erythropoietic protoporphyria (EPP) is a rare inherited disorder of the heme biosynthesis pathway resulting in the accumulation of protoporphyrins in the blood, erythrocytes, and other tissues. Because of a gene mutation in the FECH gene, ferrochelatase, the enzyme involved in the final step of heme synthesis, is deficient in these patients. Although the major symptom of this disorder is photosensitivity, rarely, it can cause progressive liver disease requiring liver transplantation (LT). However, LT is not curative and only bone marrow transplantation (BMT) can correct the underlying enzymatic defect. Because liver disease results from accumulation of protoporphyrin in the liver, LT without hematopoietic stem cell transplantation leaves the new liver at risk for similar EPP‐related damage. A handful of pediatric patients undergoing sequential LT and stem cell transplantation have been described in the literature; however, to date none has been described in detail in adults. We report a case of an adult male with EPP and liver failure who successfully underwent a sequential liver and hematopoietic stem cell transplantation (HSCT).     

The value of intravenous heme-albumin and plasmapheresis in reducing postoperative complications of orthotopic liver transplantation for erythropoietic protoporphyria

Erythropoietic protoporphyria (EPP) is marked by a deficiency of ferrochelatase, which occurs in all cells and tissues, preventing effective conversion of proto porphyrin IX to heme and thereby blocking effective feedback inhibition of heme synthesis. The major source of the excess protoporphyrin is the bone marrow. Protoporphyrin IX may accumulate, with resultant toxicity chiefly of the marrow, skin, nervous system, and liver. Orthotopic liver transplantation (OLT) is, at present, the only adequate intervention for severe liver compromise secondary to protoporphyrin deposition, but it has been complicated by severe photosensitivity and polyneuropathy. Intravenous heme and plasmapheresis have been proposed but not previously reported as means to reduce the protoporphyrin burden before liver transplantation. We report a man with EPP who underwent preoperative heme-albumin administration and plasmaphereses that led to marked reductions in plasma and erythrocyte protoporphyrin levels. His OLT was uneventful, and he developed neither polyneuropathy nor exacerbation of photosensitivity.     

Hepatitis C: indication for anti-viral therapy?

Abstract  Hepatitis C infection is a frequent indication for liver transplantation. In general, recurrent graft hepatitis is assumed to be mild, but may be the cause of lethal postoperative complications in a small patient population. Out of 500 transplants in 458 patients, 123 patients were transplanted due to hepatitis C infection (26.7 %) between September 1988 and April 1994. Cumulative 1– to 6-year patient survival was similar for patients transplanted due to hepatitis C (87.0 %) and those transplanted for other indications (86.0 %). In patients with hepatitis C virus (HCV), death, in 50 % of the cases, was related to HCV recurrence and chronic rejection. Four patients (25.0 %) died because of severe infection and multiple organ failure syndrome unrelated to HCV recurrence and chronic rejection. The incidence of retransplantation was similar in HCV (9.8 %) and other patients (8.4 %). In HCV patients, 6 of 12 retransplantations (50.0 %) were performed due to HCV recurrence and chronic rejection. Of 123 HCV patients, 45 experienced histo-logically proven recurrent graft hepatitis between 2 weeks and 5.5 years after transplantation. The incidence of acute rejection was similar in both groups. The incidence of steroid-resistant rejection was, however, higher in HCV patients (29.3 %) than in those transplanted for other indications (14.5 %; P ≤ 0.05). Furthermore, there was a significant association between acute rejection and the development of recurrent graft hepatitis. In conclusion, patients with hepatitis C may be transplanted with as good patient and graft survival rates as patients transplanted for other indications. However, the combination of recurrent graft hepatitis and chronic rejection remains the most limiting factor for some of these patients, which strengthens the necces-sity for a specific anti-viral therapy.     

Treatment of recurrent allograft dysfunction with intravenous hematin after liver transplantation for erythropoietic protoporphyria

Erythropoietic protoporphyria (EPP) is a rare inherited disorder of the heme biosynthetic pathway in which toxic levels of protoporphyrins often precipitate in the liver, leading to cirrhosis, liver failure, and the need for liver transplantation (OLT). Because the underlying enzyme defect in EPP is bone marrow derived, the risk for recurrent EPP allograft dysfunction is high. Although plasmapheresis may ameliorate acute allograft disease, strategies to maintain disease remission are needed. A 59-year-old man who underwent OLT for hepatic EPP experienced increased bilirubin and aminotransferases on postoperative day 700. Allograft biopsy demonstrated recurrent EPP. He was managed initially with plasmapheresis, hypertransfusion, and infusions of i.v. hematin. After normalization of liver tests, the hematin infusions have been given intermittently, are well tolerated, and associated with normal allograft function for nearly 2 years. This is the first case of the use of hematin given post-OLT to help achieve and maintain remission of allograft EPP disease.     

Liver transplantation for autoimmune hepatitis: fulminant versus chronic hepatitis presentation

AIMS: The aim of this study was to analyse long-term outcomes of patients with liver transplantation for autoimmune hepatitis and to determine if fulminant/subfulminant hepatic failure (FSHF) at presentation was a predictor of outcome after ortothopic liver transplantation. PATIENTS AND METHODS: Between April 1990 and October 2002, 18 patients with autoimmune hepatitis underwent 21 liver transplants. Three patients were excluded because of coexisting causes of hepatitis. Seven patients had FSHF and eight patients had chronic disease. The initial immunosuppressive regimen was cyclosporine based in 80%, and all but one patient received steroids or azathioprine. RESULTS: Mean age at time of transplant was 44.2+/-15.5 years. Patients were followed for 38.9+/-29.6 months. Five patients (33.3%) had seven episodes of acute rejection (two steroid-resistant). Three patients developed chronic rejection. One patient displayed histologically proven recurrent autoimmune hepatitis. Actuarial patient and graft survival rates at 1 and 5 years were 80% and 56% and 78.6% and 51%, respectively. No differences in the clinical characteristics of the patients, rates of acute or chronic rejection episodes, end biliary or arterial complications were observed between FSHF and chronic autoimmune hepatitis. The study suggests a better survival for autoimmune FSHF (P=.003). CONCLUSIONS: Liver transplant is indicated for patients displaying autoimmune chronic liver disease and FSHF with similar clinical courses at however, patients with FSHF at presentation had better survivals.     

Outcome of renal transplantation in fibrillary glomerulonephritis

Fibrillary glomerulonephritis (FGN) is a rare but progressive glomerular disease usually with end-stage renal disease (ESRD) developing within months or few years following the diagnosis. Little is known about the outcome of renal transplantation in patients with ESRD due to FGN. We report four patients with FGN who received renal allografts. Two patients developed recurrent FGN in their grafts. One patient was diagnosed to have recurrent FGN 9 years post-transplant, and lost his graft 4 years thereafter. Another patient had recurrent disease 2 years post-transplant but has stable graft function after 7 years. One patient died with normal renal allograft function 7 years following transplantation. The fourth patient has chronic transplant nephropathy 34 months post-transplant without evidence of recurrent FGN. A literature review revealed 10 additional patients who received 11 renal allografts due to ESRD caused by FGN. Four of these 10 patients had biopsy-proven recurrence (one patient in two subsequent grafts), but this caused graft loss only in 2 patients 56 months and 7 years post-transplant, respectively. The earliest recurrence was diagnosed 2 years post-transplant. We conclude that although the recurrence rate of FGN in renal transplants is high (around 50%), the recurrent disease has a relatively benign course and prolonged graft survival is possible.     

Combined heart–liver transplantation: a single‐center experience

Combined orthotopic heart and liver transplantation (CHLT) is a lifesaving procedure for patients with end‐stage heart–liver disease. We reviewed the long‐term outcome of patients who have undergone CHLT at the University of Bologna, Italy. Fifteen patients with heart and liver failure were placed on the transplant list between November 1999 and March 2012. The pretransplant cardiac diagnoses were familial amyloidosis in 14 patients and chronic heart failure due to chemotherapy with liver failure due to chronic hepatitis in one patient. CHLT was performed as a single combined procedure in 14 hemodynamically stable patients; there was no peri‐operative mortality. The survival rates for the CHLT recipients were 93%, 93%, and 82% at 1 month and 1 and 5 years, respectively. Freedom from graft rejection was 100%, 90%, and 36% at 1, 5, and 10 years, respectively, for the heart graft and 100%, 91%, and 86% for the liver graft. The livers of eight recipients were transplanted as a “domino” with mean overall 1‐year survival of 93%. Simultaneous heart and liver transplantation is feasible and was achieved in this extremely sick cohort of patients. By adopting the domino technique, we were able to enlarge the donor cohort and include high‐risk patients.